DOI 10.1515/jpm-2013-0238 J. Perinat. Med. 2014; 42(2): 261–262
Letter to the Editor Shailendra Kapoor*
Hepatic rupture: a rare but serious complication of HELLP syndrome Keywords: HELLP syndrome; liver; rupture. *Corresponding author: Shailendra Kapoor, Private practice, 74 Crossing Place, Mechanicsville, VA 23221, USA, Tel.: +865 567 5678, Fax: +865 678 6787, E-mail: [email protected]
To the Editor, I read with great interest the recent article by Schaarschmidt et al. [10]. Interestingly, one rare but serious complication of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome that is often overlooked is hepatic rupture. For instance, spontaneous hepatic rupture is seen in nearly 0.05% of patients with HELLP syndrome. Araujo et al. in a recent study [1] reported an incidence rate of 1 per 5346 deliveries. It usually develops as a result of rupture of a subcapsular hepatic hematoma. The subcapsular hematoma in turn develops secondary to accentuation of intrahepatic pressure due to intrahepatic vascular congestion [2]. Dilatation and detachment of the Glisson’s capsule completes the pathogenic process. HELLP syndrome in women is usually seen as a complication of preeclampsia. It is usually seen in multigravida older than 30 years. The average age of the patients is 42.5 years. A median parity of 4.5 was reported in a recent study [5]. Hepatic rupture is usually seen during pregnancy in 66% of cases; however, may manifest as much as 10 h after cesarean delivery in women with HELLP syndrome [8]. Most patients are 32–34 weeks pregnant. HELLP syndrome may also rarely be complicated by splenic rupture. The patient typically develops severe abdominal pain, usually in the right upper quadrant. This is the presenting symptom in nearly 70% of the patients [6]. Hepatic rupture has been reported in gravid women as old as 48 years of age. Severe vomiting is usually present concurrently. Dyspnea may develop rapidly. Signs and symptoms consistent with severe hypovolemic shock may appear rapidly. Multisystem organ failure may emerge rapidly.
Acute respiratory distress syndrome develops in 33% of patients [7]. Severe uterine bleeding may be seen in 33% of patients after a cesarean section. The gold standard for diagnosis of hepatic rupture is abdominal ultrasound. CT scanning as well as ultrasonography may reveal the extent of the hemoperitoneum. Rupture may be seen in one lobe only or may affect both lobes. However, overall, right lobe rupture tends to be more common in HELLP syndrome [3]. The hemoglobin may be reduced to as much as 5.8 g/dL. Serum glutamic oxaloacetic transaminase (SGOT) and serum glutamicpyruvic transaminase (SGPT) are typically elevated in 30% of patients. For instance, SGOT levels may be elevated to as much as 2500 units/L [12]. SGPT elevation up to as much as 2352 units/L has been reported. γ-Glutamyltransferase levels are typically normal. Peripheral blood examination usually reveals significant thrombocytopenia in 77.5% of the cases. For instance, platelet counts may drop to as much as 20,000. Simultaneous elevation of serum lactate dehydrogenase to as much as 4000 units/L has been reported [11]. Acute renal failure develops in 50% of the patients as is manifested by the progressively increasing creatinine levels. Liver rupture is a surgical emergency. A multidisciplinary and very aggressive approach is necessary to obtain reasonable hemostasis. Conservative medical therapy is successful in a few instances only. For instance, Voltz et al. [4] have reported success with non-surgical intervention. Urgent cesarean section and laparotomy with peri-hepatic packing are deemed necessary in pregnant women with hepatic rupture. Polypropylene meshes may also be used to control the bleeding. Omental patching by itself may be effective in some cases. Argon beam coagulation is a recent development that has shown considerable efficacy in controlling bleeding secondary to hepatic rupture [3]. Transcatheter hepatic artery embolization with gelatin particles is another therapeutic option used to limit the bleeding [5, 8]. This is more useful in unilateral lobe rupture. Reck et al. [9] have recently also reported the successful use of “fibrin glue”-coated collagen fleeces. Packed red blood cell and fresh-frozen plasma
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262 Kapoor, Liver rupture due to HELLP syndrome transfusions are usually necessary. Nearly 65% of the patients requiring surgical intervention require multiple laparotomies. As many as 13 laparotomies may be needed in a single patient [7, 8]. Blood loss during surgery may be as high as 9 L. Administration of recombinant factors such as recombinant factor VIIa may be necessary to mitigate the bleeding. The initial dose of recombinant factor VIIa is 200 μg/kg. Severe hepatic necrosis may require liver resection. Hepatic rupture may necessitate liver transplantation in severe cases [3, 4]. This should be considered as a therapeutic option only in severe hepatic failure or poor hemostasis secondary to uncontrollable bleeding. Macroscopic hepatic necrosis is another pointer toward the possible need for orthoptic hepatic transplantation. For instance, Varotti et al. [13] recently reported the case of a 43-year-old second gravida who developed bilateral
lobe rupture necessitating hepatic transplantation. The success rate of orthoptic liver transplantation is 76.9% [8]. Prolonged ICU admission is the usual course in hepatic rupture. Hospital admission may range from anywhere as less as 8 days to as long as 45 days. Pleural effusion is a common post-surgical complication and has been noted in 30% of the patients. Maternal mortality rates as high as 25–39% have been reported, whereas fetal mortality rates as high as 60% have been reported [1, 6]. Hepatic rupture is a serious complication of HELLP syndrome and requires interdisciplinary management. It should be watched out for in all patients with HELLP syndrome presenting with acute abdominal pain. Received August 29, 2013. Accepted December 13, 2013. Previously published online January 23, 2014.
References [1] Araujo AC, Leao MD, Nobrega MH, Bezerra PF, Pereira FV, Dantas EM, et al. Characteristics and treatment of hepatic rupture caused by HELLP syndrome. Am J Obstet Gynecol. 2006;195:129–33. [2] Carrel T, Huber A, Scharl HR, Gysler H, Gertsch P. Rupture of a subcapsular liver hematoma in the postpartum period associated with HELLP syndrome. Helv Chir Acta. 1990;57:29–32. [3] Dessole S, Capobianco G, Virdis P, Rubattu G, Cosmi E, Porcu A. Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature. Arch Gynecol Obstet. 2007;276:189–92. [4] Herring CS, Heywood SG, Hatjis CG. The multiple challenges in the management of a patient with HELLP syndrome, liver rupture and eclampsia. W V Med J. 2005;101:261–2. [5] Huskes KP, Baumgartner A, Hardt U, Klink F. Bilateral recurrent spontaneous rupture of the liver in HELLP syndrome. Chirurg. 1991;62:221–2. [6] Miguelote RF, Costa V, Vivas J, Gonzaga L, Menezes CA. Postpartum spontaneous rupture of a liver hematoma associated with preeclampsia and HELLP syndrome. Arch Gynecol Obstet. 2009;279:923–6. [7] Pavlis T, Aloizos S, Aravosita P, Mystakelli C, Petrochilou D, Dimopoulos N, et al. Diagnosis and surgical management of spontaneous hepatic rupture associated with HELLP syndrome. J Surg Educ. 2009;66:163–7.
[8] Peiro LZ, Salas R, Dolera Moreno C, Molla C. Spontaneous liver rupture in HELLP syndrome. Med Intensiva. 2009;33:56–7. [9] Reck T, Bussenius-Kammerer M, Ott R, Muller V, Beinder E, Hohenberger W. Surgical treatment of HELLP syndromeassociated liver rupture – an update. Eur J Obstet Gynecol Reprod Biol. 2001;99:57–65. [10] Schaarschmidt W, Rana S, Stepan H. The course of angiogenic factors in early- vs. late-onset preeclampsia and HELLP syndrome. J Perinat Med. 2013;41:511–6. [11] Shrivastava VK, Imagawa D, Wing DA. Argon beam coagulator for treatment of hepatic rupture with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Obstet Gynecol. 2006;107:525–6. [12] van de Minkelis JL, Steenvoorde P, Baranski AG. Liver rupture in a patient with HELLP syndrome successfully treated with extensive surgery combined with recombinant factor VIIa. Acta Chir Belg. 2006;106:602–4. [13] Varotti G, Andorno E, Valente U. Liver transplantation for spontaneous hepatic rupture associated with HELLP syndrome. Int J Gynaecol Obstet. 2010;111:84–5.
The authors stated that there are no conflicts of interest regarding the publication of this article.
Brought to you by | Georgetown University Authenticated Download Date | 5/27/15 7:11 AM
Letter to the Editor Shailendra Kapoor*
Hepatic rupture: a rare but serious complication of HELLP syndrome Keywords: HELLP syndrome; liver; rupture. *Corresponding author: Shailendra Kapoor, Private practice, 74 Crossing Place, Mechanicsville, VA 23221, USA, Tel.: +865 567 5678, Fax: +865 678 6787, E-mail: [email protected]
To the Editor, I read with great interest the recent article by Schaarschmidt et al. [10]. Interestingly, one rare but serious complication of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome that is often overlooked is hepatic rupture. For instance, spontaneous hepatic rupture is seen in nearly 0.05% of patients with HELLP syndrome. Araujo et al. in a recent study [1] reported an incidence rate of 1 per 5346 deliveries. It usually develops as a result of rupture of a subcapsular hepatic hematoma. The subcapsular hematoma in turn develops secondary to accentuation of intrahepatic pressure due to intrahepatic vascular congestion [2]. Dilatation and detachment of the Glisson’s capsule completes the pathogenic process. HELLP syndrome in women is usually seen as a complication of preeclampsia. It is usually seen in multigravida older than 30 years. The average age of the patients is 42.5 years. A median parity of 4.5 was reported in a recent study [5]. Hepatic rupture is usually seen during pregnancy in 66% of cases; however, may manifest as much as 10 h after cesarean delivery in women with HELLP syndrome [8]. Most patients are 32–34 weeks pregnant. HELLP syndrome may also rarely be complicated by splenic rupture. The patient typically develops severe abdominal pain, usually in the right upper quadrant. This is the presenting symptom in nearly 70% of the patients [6]. Hepatic rupture has been reported in gravid women as old as 48 years of age. Severe vomiting is usually present concurrently. Dyspnea may develop rapidly. Signs and symptoms consistent with severe hypovolemic shock may appear rapidly. Multisystem organ failure may emerge rapidly.
Acute respiratory distress syndrome develops in 33% of patients [7]. Severe uterine bleeding may be seen in 33% of patients after a cesarean section. The gold standard for diagnosis of hepatic rupture is abdominal ultrasound. CT scanning as well as ultrasonography may reveal the extent of the hemoperitoneum. Rupture may be seen in one lobe only or may affect both lobes. However, overall, right lobe rupture tends to be more common in HELLP syndrome [3]. The hemoglobin may be reduced to as much as 5.8 g/dL. Serum glutamic oxaloacetic transaminase (SGOT) and serum glutamicpyruvic transaminase (SGPT) are typically elevated in 30% of patients. For instance, SGOT levels may be elevated to as much as 2500 units/L [12]. SGPT elevation up to as much as 2352 units/L has been reported. γ-Glutamyltransferase levels are typically normal. Peripheral blood examination usually reveals significant thrombocytopenia in 77.5% of the cases. For instance, platelet counts may drop to as much as 20,000. Simultaneous elevation of serum lactate dehydrogenase to as much as 4000 units/L has been reported [11]. Acute renal failure develops in 50% of the patients as is manifested by the progressively increasing creatinine levels. Liver rupture is a surgical emergency. A multidisciplinary and very aggressive approach is necessary to obtain reasonable hemostasis. Conservative medical therapy is successful in a few instances only. For instance, Voltz et al. [4] have reported success with non-surgical intervention. Urgent cesarean section and laparotomy with peri-hepatic packing are deemed necessary in pregnant women with hepatic rupture. Polypropylene meshes may also be used to control the bleeding. Omental patching by itself may be effective in some cases. Argon beam coagulation is a recent development that has shown considerable efficacy in controlling bleeding secondary to hepatic rupture [3]. Transcatheter hepatic artery embolization with gelatin particles is another therapeutic option used to limit the bleeding [5, 8]. This is more useful in unilateral lobe rupture. Reck et al. [9] have recently also reported the successful use of “fibrin glue”-coated collagen fleeces. Packed red blood cell and fresh-frozen plasma
Brought to you by | Georgetown University Authenticated Download Date | 5/27/15 7:11 AM
262 Kapoor, Liver rupture due to HELLP syndrome transfusions are usually necessary. Nearly 65% of the patients requiring surgical intervention require multiple laparotomies. As many as 13 laparotomies may be needed in a single patient [7, 8]. Blood loss during surgery may be as high as 9 L. Administration of recombinant factors such as recombinant factor VIIa may be necessary to mitigate the bleeding. The initial dose of recombinant factor VIIa is 200 μg/kg. Severe hepatic necrosis may require liver resection. Hepatic rupture may necessitate liver transplantation in severe cases [3, 4]. This should be considered as a therapeutic option only in severe hepatic failure or poor hemostasis secondary to uncontrollable bleeding. Macroscopic hepatic necrosis is another pointer toward the possible need for orthoptic hepatic transplantation. For instance, Varotti et al. [13] recently reported the case of a 43-year-old second gravida who developed bilateral
lobe rupture necessitating hepatic transplantation. The success rate of orthoptic liver transplantation is 76.9% [8]. Prolonged ICU admission is the usual course in hepatic rupture. Hospital admission may range from anywhere as less as 8 days to as long as 45 days. Pleural effusion is a common post-surgical complication and has been noted in 30% of the patients. Maternal mortality rates as high as 25–39% have been reported, whereas fetal mortality rates as high as 60% have been reported [1, 6]. Hepatic rupture is a serious complication of HELLP syndrome and requires interdisciplinary management. It should be watched out for in all patients with HELLP syndrome presenting with acute abdominal pain. Received August 29, 2013. Accepted December 13, 2013. Previously published online January 23, 2014.
References [1] Araujo AC, Leao MD, Nobrega MH, Bezerra PF, Pereira FV, Dantas EM, et al. Characteristics and treatment of hepatic rupture caused by HELLP syndrome. Am J Obstet Gynecol. 2006;195:129–33. [2] Carrel T, Huber A, Scharl HR, Gysler H, Gertsch P. Rupture of a subcapsular liver hematoma in the postpartum period associated with HELLP syndrome. Helv Chir Acta. 1990;57:29–32. [3] Dessole S, Capobianco G, Virdis P, Rubattu G, Cosmi E, Porcu A. Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature. Arch Gynecol Obstet. 2007;276:189–92. [4] Herring CS, Heywood SG, Hatjis CG. The multiple challenges in the management of a patient with HELLP syndrome, liver rupture and eclampsia. W V Med J. 2005;101:261–2. [5] Huskes KP, Baumgartner A, Hardt U, Klink F. Bilateral recurrent spontaneous rupture of the liver in HELLP syndrome. Chirurg. 1991;62:221–2. [6] Miguelote RF, Costa V, Vivas J, Gonzaga L, Menezes CA. Postpartum spontaneous rupture of a liver hematoma associated with preeclampsia and HELLP syndrome. Arch Gynecol Obstet. 2009;279:923–6. [7] Pavlis T, Aloizos S, Aravosita P, Mystakelli C, Petrochilou D, Dimopoulos N, et al. Diagnosis and surgical management of spontaneous hepatic rupture associated with HELLP syndrome. J Surg Educ. 2009;66:163–7.
[8] Peiro LZ, Salas R, Dolera Moreno C, Molla C. Spontaneous liver rupture in HELLP syndrome. Med Intensiva. 2009;33:56–7. [9] Reck T, Bussenius-Kammerer M, Ott R, Muller V, Beinder E, Hohenberger W. Surgical treatment of HELLP syndromeassociated liver rupture – an update. Eur J Obstet Gynecol Reprod Biol. 2001;99:57–65. [10] Schaarschmidt W, Rana S, Stepan H. The course of angiogenic factors in early- vs. late-onset preeclampsia and HELLP syndrome. J Perinat Med. 2013;41:511–6. [11] Shrivastava VK, Imagawa D, Wing DA. Argon beam coagulator for treatment of hepatic rupture with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Obstet Gynecol. 2006;107:525–6. [12] van de Minkelis JL, Steenvoorde P, Baranski AG. Liver rupture in a patient with HELLP syndrome successfully treated with extensive surgery combined with recombinant factor VIIa. Acta Chir Belg. 2006;106:602–4. [13] Varotti G, Andorno E, Valente U. Liver transplantation for spontaneous hepatic rupture associated with HELLP syndrome. Int J Gynaecol Obstet. 2010;111:84–5.
The authors stated that there are no conflicts of interest regarding the publication of this article.
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